[Sinus histiocytosis with massive lymphadenopathy: a new case in an adult with anti factor VIII antibody (author's transl)].
The case history of a 71 year old french man with a lethal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman's Syndrome) is reported. The histological features were typical but the clinical course was remarkable because of the predominant expression was a life threatening hemorrhagic disorder closely linked to a circulating anticoagulant (anti-factor VIII). The bringing to light in a picture of hyperimmunity of a self antibody and a high anti-EBV titer suggest on lymphocyte T deficit could be important in the development of the sinus histiocytosis.
D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D005169:Factor VIII / Q000276:immunology*; D006801:Humans; D008206:Lymphatic Diseases / Q000276:immunology* / Q000473:pathology; D008297:Male; D013577:Syndrome
D001323:Autoantibodies; D005169:Factor VIII
Maladie de Rosaï: forme grave chez un adulte avec présence d'un anticorps antifacteur VIII.